Non-traumatic osteonecrosis is a frequent complication in patients with autoimmune disorders, though its prevalence varies markedly depending upon the type of disorder. Understanding the causes of this difference can help uncover the underlying pathophysiology of osteonecrosis and guide the development of effective preventive and therapeutic strategies.

In this perspective study, we reviewed available databases, including PubMed, Cochrane Library, Scopus, and Web of Science, to explore why the risk of osteonecrosis varies among different autoimmune disorders. Is this variation primarily due to the disease's pathophysiology, the use of medications such as corticosteroids, or a combination of both? If both factors are involved, what is the extent of each contribution in this context?

Non-traumatic osteonecrosis is often induced by an interaction between disease pathophysiology and corticosteroid use. In patients with different autoimmune disorders but an identical history of corticosteroid use, the risk of osteonecrosis is influenced by how the underlying pathophysiology compromises bone health. In autoimmune disorders with multiple adverse effects on bone, such as SLE (systemic lupus erythematosus), there is a much higher risk of osteonecrosis compared to disorders with minimal impact on bone health, such as celiac disease and MS (multiple sclerosis).

Being defined as an autoimmune, chronic pathology, frequently encountered in any age group, but especially in pediatrics, celiac disease (also called gluten enteropathy), is gaining more and more ground in terms of diagnosis, but also interest in research. The data from the literature of the last decades attest the chameleonic way of its presentation, there may be both classic onset symptoms and atypical symptoms. Given the impact played by celiac disease, especially in the optimal growth and development of children, the current narrative review aims to highlight the atypical presentation methods, intended to guide the clinician towards the inclusion of the pathology in the differential diagnosis scheme. To these we add the summary presentation of the general data and therapeutic lines regarding the underlying condition and the existing comorbidities. In order to place the related information up to date, we performed a literature review of the recent articles published in international databases. We bring forward the current theories and approaches regarding both classic celiac disease and its atypical manifestations. Among these we note mainly constitutional, skin or mucous, bone, neuro-psychic, renal, reproductive injuries, but also disorders of biological constants and association with multiple autoimmunities. Knowing and correlating them with celiac disease is the key to optimal management of patients, thus reducing the subsequent burden of the disease.

Acquired coagulopathy is a rare but challenging diagnosis for pediatric emergency physicians. Although the coagulopathy usually presents with mild skin and mucosal hemorrhages, it also can lead to life-threatening events. Thus, accurate interpretation of hints obtained from a detailed history, physical examination, and laboratory findings is essential for the prompt diagnosis and management. This case demonstrates an uncommon cause of coagulopathy; celiac disease that presented with spontaneous bruises and ecchymosis in an adolescent.

Celiac disease is a common multisystemic autoimmune disorder. It is now increasingly recognized that it may present with extraintestinal manifestations which contribute to the difficulty in its diagnosis. The objective of this scholarly review was to examine the extraintestinal ENT manifestations of celiac disease and its pathophysiology and management, in order to highlight that some patients with celiac disease may present initially to the otolaryngologist. Improving awareness of celiac disease among otolaryngologists may aid in the correct diagnosis and correct management plan.

A literature review was conducted using the PubMed database to identify original articles related to celiac disease and ENT manifestations between the years 2000 and 2020. The search was performed using the search string: ("coeliac disease" OR "celiac disease") AND ("ENT manifestations" OR "hearing loss" OR "epistaxis" OR "nasal septal perforation" OR "obstructive sleep apnoea" OR "vertigo" OR "tonsillitis" OR "sinusitis"). Only articles written in English were reviewed.

A total of 17 papers met the inclusion criteria. Extraintestinal ENT manifestations of celiac disease include sensorineural hearing loss, obstructive sleep apnea, nasal septal perforation, epistaxis, and vertigo with nystagmus. Sensorineural hearing loss, obstructive sleep apnea, nasal septal perforation, vertigo, and nystagmus are thought to result from immunologically mediated mechanisms, with intestinal malabsorption resulting in epistaxis.

Celiac disease can cause extraintestinal ENT manifestations and requires a high index of suspicion from the otolaryngologist to diagnose and suitably manage. A gluten-free diet may result in sufficient symptom resolution for most manifestations. Sensorineural hearing loss due to celiac disease appears to be progressive and permanent and may require frequent audiological monitoring.

Symptomatic coagulopathies in celiac disease (CD) are rare. Here, we report a profound case of coagulopathy in a celiac. A 66-year old female without liver disease or anti-coagulation therapy presented with multiple ecchymoses, guaiac positive melanic stool, and a recent 4.5kg weight loss. Laboratory values included hemoglobin, 3.8g/dL; MCV, 66 fL; serum iron, 17μg/dL; platelet count, 580K/μL; white count, 14.2K/μL, and vitamin D,<5.0ng/mL. Additional values included partial thromboplastin time (PTT), >200s; prothrombin time (PT), >150s; INR, 20.5, putting her at extreme risk of bleeding. Vitamin K deficiency was assumed. The patient was given two units of fresh frozen plasma, packed red cells, and vitamin K intravenously. Endoscopy and biopsies demonstrated duodenal mucosal atrophy with cobblestoning, erosive gastritis, flattened duodenal villi and numerous intraepithelial lymphocytes. Transglutaminase serology demonstrated IgA TTG>100 U/mL (normal<3U/mL), confirming a diagnosis of CD. The patient's coagulopathy resolved within two days following admission. This case underscores the importance of CD testing in all patients with coagulopathies of unknown origin. Although coagulopathy is an uncommon presentation of CD, in extreme cases such as this, it has the potential to be life-threatening.

BACKGROUND Celiac crisis is an uncommon but critical complication of celiac disease (CD) manifesting with copious diarrhea, dehydration, and severe metabolic imbalances. Celiac crisis occurring in individuals who have been formerly diagnosed with CD and displaying severe coagulopathy is tremendously rare. CASE REPORT We report a case of a 76-year-old male, previously diagnosed with CD and non-compliant with gluten free diet, who presented with severe coagulopathy manifesting as gastrointestinal bleeding in addition to other features of celiac crisis, including severe diarrhea, dehydration, metabolic acidosis, electrolyte disturbances, and renal dysfunction. Esophagogastroduodenoscopy revealed flattened mucosa and mucosal nodularity in the duodenum. Duodenal biopsies exhibited active chronic inflammation with intraepithelial lymphocytosis and subtotal villous blunting. The patient was diagnosed with celiac crisis and treatment with vitamin K, parenteral nutrition, and steroids was commenced. After initial clinical improvement, a gluten-free diet was implemented with complete resolution of symptoms. CONCLUSIONS Though celiac crisis typically presents in patients with undiagnosed CD, it should be considered in patients who have been previously diagnosed CD but who are non-compliant with gluten free diet. Severe coagulopathy, though extremely rare, can be a feature of celiac crisis and should be consider when encountered in a patient with history of steatorrhea and gastrointestinal bleeding.

Celiac disease (CD) is an autoimmune response to ingestion of gluten protein, which is found in wheat, rye, and barley grains, and results in both small intestinal manifestations, including villous atrophy, as well as systemic manifestations. The main treatment for the disease is a gluten-free diet (GFD), which typically results in the restoration of the small intestinal villi, and restoration of other affected organ systems, to their normal functioning. In an increasing number of recently published studies, there has been great interest in the occurrence of alterations in the cardiovascular system in untreated CD. Herein, published studies in which CD and cardiovascular terms appear in the title of the study were reviewed. The publications were categorized into one of several types: (1) articles (including cohort and case-control studies); (2) reviews and meta-analyses; (3) case studies (one to three patient reports); (4) letters; (5) editorials; and (6) abstracts (used when no full-length work had been published). The studies were subdivided as either heart or vascular studies, and were further characterized by the particular condition that was evident in conjunction with CD. Publication information was determined using the Google Scholar search tool. For each publication, its type and year of publication were tabulated. Salient information from each article was then compiled. It was determined that there has been a sharp increase in the number of CD - cardiovascular studies since 2000. Most of the publications are either of the type "article" or "case study". The largest number of documents published concerned CD in conjunction with cardiomyopathy (33 studies), and there have also been substantial numbers of studies published on CD and thrombosis (27), cardiovascular risk (17), atherosclerosis (13), stroke (12), arterial function (11), and ischemic heart disease (11). Based on the published research, it can be concluded that many types of cardiovascular issues can occur in untreated CD patients, but that most tend to resolve on a GFD, often in conjunction with the healing of small intestinal villous atrophy. However, in some cases the alterations are irreversible, underscoring the need for CD screening and treatment when cardiovascular issues arise of unknown etiology.

Celiac disease, an autoimmune disease once thought to be uncommon, is now being increasingly identified. Our improved diagnostic modalities have allowed us to diagnose more and more patients with atypical symptoms who improve on gluten-free diet (GFD). We discuss here the latest findings regarding the various hematological manifestations of celiac disease and their management. Anemia remains the most common hematological manifestation of celiac disease due to many mechanisms, and can be the sole presenting symptom. Other manifestations include thrombocytosis and thrombocythemia, leukopenia, thromboembolism, increased bleeding tendency, IgA deficiency, splenic dysfunction, and lymphoma. The diagnosis of celiac disease should always be kept in mind when a patient presents with unexplained and isolated hematological finding. Once diagnosed, patients should adhere to GFD and be educated about the potential complications of this disease. We herein present an algorithm for adequate management and follow-up.

Short bowel syndrome (SBS) occurs after massive intestinal resection, and parenteral nutrition (PN) therapy may be necessary even after a period of adaptation. The purpose of this study was to determine the vitamin status in adults with SBS receiving intermittent PN.

The study was conducted on hospitalized adults with SBS who were receiving intermittent PN therapy (n = 8). Nine healthy volunteers, paired by age and sex, served as controls. Food ingestion, anthropometry, plasma folic acid, and vitamins B(12), C, A, D, E, and K were evaluated.

The levels of vitamins A, D, and B(12) in both groups were similar. SBS patients presented higher values of folic acid (21.3 ± 4.4 vs 14.4 ± 5.2, P = .01) and lower values of vitamin C (0.9 ± 0.4 vs 1.2 ± 0.3 mg/dL, P = .03), α-tocopherol (16.3 ± 3.4 vs 24.1 ± 2.7 µmol/L, P < .001), and phylloquinone (0.6 ± 0.2 vs 1.0 ± 0.5 nmol/L, P < .03). Eight-seven percent of patients had vitamin D deficiency, and all patients presented with serum vitamin E levels below reference values.

Despite all efforts to offer all the nutrients mentioned above, SBS patients had lower serum levels of vitamins C, E, and K, similar to those observed in patients on home PN. These findings suggest that the administered vitamins were not sufficient for the intermittent PN scheme and that individual adjustments are needed depending on the patient's vitamin status.

A 36-year-old gentleman presented with 6 months of poor energy, tingling in fingers and weight loss with a change in bowel habit. He appeared cachectic and had clubbing, demineralisation of teeth, pectus carinatus, kyphosis, spinal tenderness, proximal muscle weakness and generalised muscle atrophy. Chvostek's and Trosseau's signs were positive. His haemoglobin (Hb) was 8.7 g/dl, MCV 64.7 fl with low iron. Calcium corrected was 1.30 nmol/l, parathyroid hormone 440.4 ng/l, vitamin D <12.5 nmol/l; INR was 2.7 with coagulation inhibitor studies negative. Radiographs of spine and pelvis commented on osteopenia with thoracic kyphosis and mild anterior wedging of thoracic vertebrae. Antitissue transglutaminase was 145 U/ml, and antiendomysial antibodies were positive. An oesophagogastroduodenoscopy was consistent with coeliac disease. A diagnosis of osteomalacia and coagulopathy secondary to coeliac disease was made. The hypocalcaemia was treated with calcium gluconate infusions with symptomatic relief. Coagulopathy was treated with vitamin K intravenously with normalisation of INR. Following treatment with coeliac diet, calcium slowly normalised.

This study examined the changes of activities of vitamin K-dependent clotting factors (VKDCF) under various pathological conditions and explored the relationship between acquired deficiency of VKDCFs and hemorrhage. Clinical data of 35 patients who were diagnosed as having acquired deficiency of VKDCF were retrospectively analyzed. Coagulation factors involved in the intrinsic and extrinsic pathways were detected in these patients and 41 control subjects. The results showed that the average activities of VKDCFs were decreased in the patients in comparison to the control subjects and significantly increased after treatment of these patients with vitamin K and blood products. Multivariate regression analysis indicated that decreased activity of VKDCF was not an independent risk factor for bleeding disorders owing to deficiency or metabolic disturbance of vitamin K. It was concluded that acquired deficiency of VKDCF occurs under a variety of pathologic conditions and is closely associated with hemorrhagic events. Administration of vitamin K and transfusion of blood products containing high concentrations of VKDCFs helps alleviate the hemorrhagic diseases.